To facilitate more accurate diagnosis and suggest new approaches to therapy of heritable dissorders of connective tissue by studies designed to define the pathogenesis of the disease and allow classification according to primary biochemical defect rather than by clinical phenotype. To study those diseases with probable primary or secondary abnormalities in collagen biosynthesis which have similar phenotypes. These include the various forms of the Marfan Syndrome, Homocystinuria and patients with sickle cell disease with Marfanoid habitus as one phenotype, the various forms of Ehlers-Danlos syndrome as another. Finally X-linked cutis laxa and Menke's Kinky Hair syndrome will be studied as models in which the presumed biochemical defect, lysyl oxidase deficiency, requires further characterization. To screen for probable disorders of collagen biosynthesis by studying patients who have increased soluble skin collagen and to elucidate the mechanism for this by in vitro studies with fibroblast cultures and model systems as well as by direct characterization of collagen from skin biopsies.